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STAFF ROUND STAFF ROUND PRESENTATION PRESENTATION BY BY Prof. Dr. Gamal Esmat Prof. Dr. Gamal Esmat unit unit

Cholestasis gamal e smat

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STAFF ROUNDSTAFF ROUND PRESENTATIONPRESENTATION

BYBY

Prof. Dr. Gamal Esmat unitProf. Dr. Gamal Esmat unit

Prof. Dr. Gamal Esmat unitProf. Dr. Gamal Esmat unit

PERSONAL HISTORY:PERSONAL HISTORY: • Female patient, 28 years old, Female patient, 28 years old, married 3 years agomarried 3 years ago

mother of one girl 2 years old, housewifemother of one girl 2 years old, housewife..

• Born and living in Giza, with history of contact with Born and living in Giza, with history of contact with canal water but she did not receive antischistosomal ttt.canal water but she did not receive antischistosomal ttt.

• No special habits of medical importance.No special habits of medical importance.

• Menarche at 13 with history of OCPs Menarche at 13 with history of OCPs Kind if possibleKind if possible intake intake for 2 months before the onset of her illness. Menses are for 2 months before the onset of her illness. Menses are regular with average amount 4/28.regular with average amount 4/28.

COMPLAINT:COMPLAINT:

Yellowish discoloration of sclera 5 Yellowish discoloration of sclera 5 months before hospital admissionmonths before hospital admission

PRESENT HISTORY:PRESENT HISTORY:

• The condition started The condition started 5 months5 months ago adjust ago adjust timetime by gradually progressive by gradually progressive jaundicejaundice, dark , dark colored urine but she did not notice change in colored urine but she did not notice change in color of stools and no itching.color of stools and no itching.

The condition was not associated with:The condition was not associated with:

• Fever, rigors ,abdominal pain or vomiting.Fever, rigors ,abdominal pain or vomiting.

• Bleeding tendency, haematemesis, melena, Bleeding tendency, haematemesis, melena, ascites, LL edema or DCL.ascites, LL edema or DCL.

• History suggestive of anaemia or blood History suggestive of anaemia or blood transfusion.transfusion.

• Joint pains or skin rash.Joint pains or skin rash.

• Weight loss or perception of body masses.Weight loss or perception of body masses.

• History of traveling abroadHistory of traveling abroad

• During hospital stay she had During hospital stay she had degreedegree trauma to RT foot (torsion) trauma to RT foot (torsion) fracture base of the 5fracture base of the 5thth metatarsal metatarsal bone cast was applied bone cast was applied for how long? for how long?

and no medications were given.and no medications were given.

PAST HISTORY:PAST HISTORY:

• No diabetes mellitus or systemic hypertension.No diabetes mellitus or systemic hypertension.

• History of Caesarian section 2 years ago with no History of Caesarian section 2 years ago with no postoperative complications and no blood transfusion.postoperative complications and no blood transfusion.

• History of drug intake (intermittent intake of NSAIDs History of drug intake (intermittent intake of NSAIDs indication and dosage on the averageindication and dosage on the average and OCPS for 2 months before and OCPS for 2 months before onset of illness).onset of illness).

FAMILY HISTORY:FAMILY HISTORY:

No similar conditions in the family.No similar conditions in the family.

EXAMINATION

GENERAL EXAMINATION:GENERAL EXAMINATION:

• The patient is fully conscious, well oriented to time, The patient is fully conscious, well oriented to time, place and persons, of average mood and mentality.place and persons, of average mood and mentality.

• Average body weight.Average body weight.BMIBMI

• Blood pressure: 120/80.Blood pressure: 120/80.

• Pulse: 80/min, regular, equal on both sides, with intact Pulse: 80/min, regular, equal on both sides, with intact peripheral pulsations.peripheral pulsations.

• Temperature: afebrile all through hospital stay.Temperature: afebrile all through hospital stay.

HEAD AND NECK:HEAD AND NECK:

• Jaundice.Jaundice.• No pallor or cyanosis.No pallor or cyanosis.• No palpable lymph nodes.No palpable lymph nodes.• Trachea central. Trachea central. • Thyroid gland not felt.Thyroid gland not felt.• Neck veins are not congested.Neck veins are not congested.

UPPER LIMBS:UPPER LIMBS:

• No palmar erythemaNo palmar erythema, , spider naevi, flapping spider naevi, flapping tremors itching marks or clubbingtremors itching marks or clubbing

• Ecchymotic patches at sites of injections.Ecchymotic patches at sites of injections.

LOWER LIMBS:LOWER LIMBS:

No edema.No edema.

CVS, CHEST & NEUROLOGICAL CVS, CHEST & NEUROLOGICAL EXAMINATION:EXAMINATION:

FREEFREE

ABDOMINAL EXAMINATION:ABDOMINAL EXAMINATION:

• Normal shape and contour.Normal shape and contour.

• Right Subcostal angle Right Subcostal angle

• No No Divarication of the recti.Divarication of the recti.

• Umbilicus: normal shape and position with no Umbilicus: normal shape and position with no impulse on cough.impulse on cough.

• PfennestialPfennestial incision 10 cms healed by 1ry intention. incision 10 cms healed by 1ry intention.

• No abdominal wall veins or pigmentationsNo abdominal wall veins or pigmentations ..

• SUPERFICIAL PALPATION:SUPERFICIAL PALPATION: No tenderness, rigidity or masses detected.No tenderness, rigidity or masses detected.

• LIVER:LIVER:- - Upper borderUpper border: 5: 5thth space mid clavicular line. space mid clavicular line.- - Lower borderLower border::

RT LOBE not feltRT LOBE not felt LT LOBE is detected 5 cms below LT LOBE is detected 5 cms below

xiphisternum by light percussionxiphisternum by light percussion

• SPLEENSPLEEN:: felt 3 cms below left costal margin (firm - felt 3 cms below left costal margin (firm -sharpsharp border – not tender border – not tender

• No ascites by shifting dullness.No ascites by shifting dullness.

To summarizeTo summarize

• 28 years old female patient28 years old female patient

• Jaundice for 5 monthsJaundice for 5 months

• No history suggestive of hemolysisNo history suggestive of hemolysis

• No history suggestive of LCFNo history suggestive of LCF

• History of OCPs History of OCPs

• SplenomegalySplenomegaly

INVESTIGATIONSINVESTIGATIONS

• URINE ANALYSIS:URINE ANALYSIS:

Free apart from bile pigments , Free apart from bile pigments , urobilinogen Ca oxalate crystals .urobilinogen Ca oxalate crystals .

• STOOL ANALYSIS:STOOL ANALYSIS: FREE.FREE.

CBC:CBC:

WBCS: 7.5x10³/µl B: 1 E: 2 STAFF: 1 SEG: 65 L: 28 M: 6

HGB: 11.1g/dl MCV: 99.9 fl MCH: 34.0pg MCHC: 34.0g/dl PLT:PLT:172172x10³/µLx10³/µL

• Mild normocytic normochromic anaemia with anisopokilocytosis

• ESR : 1st hr 50

LIVER BIOCHEMICAL PROFILE: LIVER BIOCHEMICAL PROFILE: ddatesates

• BILBIL UDCA NSAIDSUDCA NSAIDS

T 1.2 23 14 21.5 21 T 1.2 23 14 21.5 21

D 0.6 15 9 14.2 13 D 0.6 15 9 14.2 13

• AST:AST: 84 686 388 101084 686 388 1010 498 498

• ALT:ALT: 67 176 123 67 176 123 264 264 156 156

• ALP:ALP: 360 360 250250

• GGT:GGT: 126 96 126 96

• T. P:T. P: 7.6 7.6

• ALB:ALB: 2.8 3 2.8 3

Vit.KVit.K• PC:PC: 33% 71% 33% 71%

• Serum sodium: 138 Serum sodium: 138 (132.0-145 mmol/L)(132.0-145 mmol/L)

• Serum potassium: 4.4 Serum potassium: 4.4 (3.6-5.5 mmol/L)(3.6-5.5 mmol/L)

• Creatinine: 0.5 Creatinine: 0.5 (0.7-1.2 mg/dl)(0.7-1.2 mg/dl)

• Urea: 15 Urea: 15 (10-50 mg/dl)(10-50 mg/dl)

• FBS:FBS: 84 84 (60 -110 g/dl )(60 -110 g/dl )

ABDOMINAL ULTRASOUND:ABDOMINAL ULTRASOUND:• LIVER:LIVER: Average in size, coarse echopattern, regular surface, attenuated hepatic veins, Average in size, coarse echopattern, regular surface, attenuated hepatic veins,

no focal lesions or IHBR dilatation. PV is not dilated.no focal lesions or IHBR dilatation. PV is not dilated.• GALL BLADDERGALL BLADDER:: Contracted but it is seen Contracted but it is seen calcular.calcular. CBD is not dilated. CBD is not dilated.

• SPLEEN:SPLEEN: Enlarged (Enlarged (17cms17cms), homogenous .), homogenous .• KIDNEYS:KIDNEYS: Both showed average size, parenchymal thickness, normal echogenicity, no Both showed average size, parenchymal thickness, normal echogenicity, no

calculi or back pressure changes.calculi or back pressure changes.• MIDLINE structure: MIDLINE structure: free.free.• NO ASCITESNO ASCITES

• CONCLUSION:CONCLUSION:– Chronic parenchymatous liver diseaseChronic parenchymatous liver disease– SplenomegalySplenomegaly

HAV( IgM –IgG) HAV( IgM –IgG) NEGATIVE NEGATIVE

HBsAg HBsAg NEGATIVE NEGATIVE Anti-HBs Anti-HBs NEGATIVENEGATIVE Anti-HBc (IgM-IgG) Anti-HBc (IgM-IgG) NEGATIVENEGATIVE

Anti-HCV Anti-HCV NEGATIVE NEGATIVE PCR FOR HCV RNA PCR FOR HCV RNA NEGATIVE NEGATIVE

ANA ANA NEGATIVENEGATIVE SMA SMA NEGATIVENEGATIVE LKMA LKMA NEGATIVENEGATIVE AMA AMA NEGATIVENEGATIVE

• SERUM COPPER: 80mcg (70-160)SERUM COPPER: 80mcg (70-160)

• 24 hrs urinary copper : 24 hrs urinary copper : 880mcg/24hrs880mcg/24hrs

• Serum ceruloplasmin : 0.28 g/l (0.2-0.6)Serum ceruloplasmin : 0.28 g/l (0.2-0.6)

• Serum Fe: 36 mcg/dl (37-158)Serum Fe: 36 mcg/dl (37-158)

• Serum FERRITIN: 43.5ng (6-159)Serum FERRITIN: 43.5ng (6-159)

• Serum Serum αα--1 antitrypsin: 172mg/dl (90-200)1 antitrypsin: 172mg/dl (90-200)

SLIT LAMP EXAMINATIONSLIT LAMP EXAMINATION

NO KAYSER FLEISCHER RINGNO KAYSER FLEISCHER RING

UPPER GIT ENDOSCOPY:UPPER GIT ENDOSCOPY:• EsophagusEsophagus: Incompetent cardia: Incompetent cardia

• Stomach:Stomach: The whole gastric mucosa is hyperaemic and The whole gastric mucosa is hyperaemic and edematous( mosaic pattern)edematous( mosaic pattern)

• Pyloric ringPyloric ring: : Rounded and active.Rounded and active.

• DuodenumDuodenum:: Free down to the second part. Free down to the second part.

CONCLUSION:CONCLUSION: Congestive gastropathyCongestive gastropathy

LIVER BIOPSYLIVER BIOPSYGROSS:GROSS: Partially fragmented core totally submittedPartially fragmented core totally submittedMICROSCOPIC:MICROSCOPIC: Hepatic tissue exhibiting widely expanded portal Hepatic tissue exhibiting widely expanded portal

tract showing marked fibrosis ,small bile duct tract showing marked fibrosis ,small bile duct proliferation and wide exudation of lymphocytes proliferation and wide exudation of lymphocytes together with neutrophils ,eosinophils,some plasma together with neutrophils ,eosinophils,some plasma cells some histiocytes. Focal inflammatory-cells some histiocytes. Focal inflammatory-lymphocytic bile duct injury is noted . There is little lymphocytic bile duct injury is noted . There is little parenchyma (in the biopsy) showing reactive atypia parenchyma (in the biopsy) showing reactive atypia with no steatosis or conspicuous nuclear with no steatosis or conspicuous nuclear glycogenation. glycogenation.

Staining for copper shows Staining for copper shows mild copper overloadmild copper overload..

CONCLUSION:CONCLUSION:

Portal florid fibroinflammatory pattern with Portal florid fibroinflammatory pattern with bile ductular proliferation with mild copper bile ductular proliferation with mild copper overload.overload.

1ry BILIARY CIRRHOSIS 1ry BILIARY CIRRHOSIS

V.SV.S

DRUG HEPATITISDRUG HEPATITIS

THANK YOUTHANK YOU